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Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

Published online by Cambridge University Press:  10 January 2020

Annika Öhman ,
Milad El-Segaier ,
Gunnar Bergman ,
Katarina Hanseus ,
Torsten Malm ,
Boris Nilsson ,
Aldina Pivodic ,
Annika Rydberg ,
Sven-Erik Sonesson  and
Mats Mellander Open the ORCID record for Mats Mellander [Opens in a new window]
Annika Öhman
Affiliation:
Department of Paediatric Cardiology, Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Sweden
Milad El-Segaier
Affiliation:
Department of Paediatric Cardiology, Skåne University Hospital, Lund, Sweden
Gunnar Bergman
Affiliation:
Department of Paediatric Cardiology, Karolinska University Hospital, Stockholm, Sweden
Katarina Hanseus
Affiliation:
Department of Paediatric Cardiology, Skåne University Hospital, Lund, Sweden
Torsten Malm
Affiliation:
Paediatric Cardiac Surgery Unit, Children’s Hospital, Skåne University Hospital, Lund, Sweden
Boris Nilsson
Affiliation:
Paediatric Cardiac Surgery Unit, Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden
Aldina Pivodic
Affiliation:
Statistiska Konsultgruppen, Gothenburg, Sweden
Annika Rydberg
Affiliation:
Department of Clinical Sciences, Paediatrics, Umeå University, Umeå, Sweden
Sven-Erik Sonesson
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
Mats Mellander*
Affiliation:
Department of Paediatric Cardiology, Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Sweden
*
Author for correspondence: Associate Professor M. Mellander, MD, PhD, Department of Paediatric Cardiology, Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, Rondvägen 10, 41650Gothenburg, Sweden. Tel: 0046705530606; Fax: 004631845029; E-mail: [email protected]
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Abstract

Background:

Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia.

Methods:

Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993–2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016.

Results:

The 1993–2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome.

Conclusion:

The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.

Keywords

Hypoplastic left heart syndrometransplantation-free survivalrisk factorsinter-stage mortality
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 3 , March 2020 , pp. 353 - 360
Copyright
© Cambridge University Press 2020

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